Skip to main content
SpringerLink
Log in

Petrified ears in a patient with Keutel syndrome: temporal bone CT findings

  • Case Report
  • Published:
Pediatric Radiology Aims and scope Submit manuscript

Abstract

We present unusual imaging findings of petrified ears in a 9-year-old girl with Keutel syndrome. The patient presented for a temporal bone study for hearing loss. CT scan showed middle and inner ear abnormalities along with extensive and unsuspected calcification of the external ears and ossicular ligaments. On further investigation, the patient was found to have diffuse cartilage calcification in the larynx and tracheobronchial tree, brachytelephalangism and peripheral pulmonary stenosis suggestive of Keutel syndrome. Confirmation was obtained by mutation analysis.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

Similar content being viewed by others

References

  1. Keutel J (1972) A new autosomal recessive syndrome: peripheral pulmonary stenosis, brachytelephalangism, neural hearing loss and abnormal cartilage calcification/ossification. In: Bergsma D, Lenz W (eds) The cardiovascular system. Liss (for the National Foundation-March of Dimes), New York, pp 60–68

  2. Cormode EJ, Dawson M, Lowry RB (1986) Keutel syndrome: clinical report and literature review. Am J Med Genet 24:289–294

    Article  PubMed  CAS  Google Scholar 

  3. Teebi AS, Lambert DM, Kaye GM, et al (1998) Keutel syndrome: further characterization and review. Am J Med Genet 77:182–187

    Article  PubMed  Google Scholar 

  4. Ziereisen F, De Munter C, Perlmutter N (1993) The Keutel syndrome. Report of a case and review of the literature. Pediatr Radiol 23:314–315

    Article  PubMed  CAS  Google Scholar 

  5. Khosroshahi HE, Uluoglu O, Olgunturk R, et al (1989) Keutel syndrome: a report of four cases. Eur J Pediatr 149:188–191

    Article  PubMed  CAS  Google Scholar 

  6. Lou G, Ducy P, McKee MD, et al (1997) Spontaneous calcification of arteries and cartilage in mice lacking matrix GLA protein. Nature 386:78–81

    Article  PubMed  Google Scholar 

  7. Hur DJ, Raymond GV, Kahler SG, et al (2005) A novel MGP mutation in a consanguineous family: review of the clinical and molecular characteristics of Keutel syndrome. Am J Med Genet 135A:36–40

    Article  Google Scholar 

  8. Miller S (2003) Brachytelephalangy with sparing of the fifth distal phalanx: a feature highly suggestive of Keutel syndrome. Pediatr Radiol 33:186–189

    Article  PubMed  Google Scholar 

  9. Stites PC, Boyd AS, Zic J (2003) Auricular ossificans (ectopic ossification of the auricle). J Am Acad Dermatol 49:142–144

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Diagnostic Imaging, The Hospital for Sick Children, 555 University Ave., Toronto, ON, M5G 1X8, Canada

    Hemant Parmar, Susan Blaser & Shi-Joon Yoo

  2. Division of Clinical and Metabolic Genetics, The Hospital for Sick Children, Toronto, ON, Canada

    Sheila Unger

  3. Institute of Human Genetics and Anthropology, University of Freiburg, Freiburg, Germany

    Sheila Unger

  4. Department of Otolaryngology, The Hospital for Sick Children, Toronto, ON, Canada

    Blake Papsin

Authors
  1. Hemant Parmar
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Susan Blaser
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Sheila Unger
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Shi-Joon Yoo
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Blake Papsin
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Hemant Parmar.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Parmar, H., Blaser, S., Unger, S. et al. Petrified ears in a patient with Keutel syndrome: temporal bone CT findings. Pediatr Radiol 36, 241–243 (2006). https://doi.org/10.1007/s00247-005-0036-7

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00247-005-0036-7

Keywords

Search

Navigation